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• W103471094 abstract "OBJECTIVE: To investigate the differences in clinical signs and symptoms between progressive supranuclear palsy with cerebellar ataxia (PSP-C) and multiple system atrophy-cerebellar type (MSA-C). BACKGROUND: Several clinical variants of PSP have been identified. We have defined a variant of PSP, PSP-C. Because patients with PSP-C develop cerebellar ataxia as the initial and principal symptom, they might be clinically misdiagnosed as having MSA-C. Because a specific biomarker of PSP has not yet been found, signs and symptoms are useful for making a diagnosis. We compared the signs and symptoms in PSP-C with those in MSA-C in pathologically proven patients. DESIGN/METHODS: We reviewed the medical records of four consecutive Japanese patients with pathologically proven PSP-C as well as 11 consecutive Japanese patients with pathologically proven MSA-C. We recorded the presence or absence of clinical features both early in (within two years of disease onset) and at any time during the disease course in these patients. We compared the signs and symptoms early in the disease course in PSP-C patients with those in MSA-C patients. RESULTS: All of the PSP-C patients (4/4, 100%) developed cerebellar ataxia and falls early in their disease course. Additionally, three of them (3/4, 75%) developed supranuclear vertical gaze palsy and postural instability. All of the PSP-C patients developed cardinal signs of PSP during the disease course. In contrast, in MSA-C patients, three of the 11 patients (27.3%) developed falls and none (0/10,0%) developed supranuclear vertical gaze palsy early in the disease course. The frequencies of falls and development of supranuclear vertical gaze palsy early in the disease course were higher in PSP-C than in MSA-C (p=0.026 and p=0.011). CONCLUSIONS: Early falls and supranuclear vertical gaze palsy predict the diagnosis of PSP-C in patients with cerebellar ataxia. Such a symptom and sign may help distinguish between PSP-C and MSA-C early in the disease course. Disclosure: Dr. Kanazawa has nothing to disclose. Dr. Shimohata has nothing to disclose. Dr. Tada has nothing to disclose. Dr. Onodera has nothing to disclose. Dr. Takahashi has nothing to disclose. Dr. Nishizawa has nothing to disclose." @default.
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• W103471094 date "2013-02-12" @default.
• W103471094 modified "2023-09-23" @default.
• W103471094 title "Early Clinical Features in Japanese Patients with Pathologically Proven Progressive Supranuclear Palsy with Cerebellar Ataxia (P04.163)" @default.
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